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stigmata Flashcards

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1
Q

DMS- cutaneous

A
  • heliotrope, V shaped photosensitive poikiloderma, shawl sign,
  • holster, flagellate, photodistributed erythema,calcinosis cutis
  • hands: gottrons sign/papules( over IPJ vs LE ), nailfold telangiectasia/ragged cuticle, mechanic’s hands,Raynaud’s
  • scalp poikiloderma/alopecia
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2
Q

ClassX: DMS

A

bullous ,amyopathic(26%), antisynthetase S

juvenile

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3
Q

antisynthetase S features, prevalence and significance?

A

30%,
raynaud’s, mechanics hands, ILD,
anti Jo 1 ab

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4
Q

juvenile DMS

A

calcinosis cutis, arthritis, RBBB, conduction)

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5
Q

DMS systemic

A
  1. myositis/Esophageal/ILD/carditis;
  2. malignancy 30% ovarian worldwide, NPC locally(50% of 20%), (LN,epistaxis, cranial nerve palsy, abdo masses/ascites, breast pelvic PR exam/LOW/BH)
  3. overlap syndrome: SLe, scleroderma,MCTD, RA/Sjogrens
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6
Q

scleroderma cutaneous

A

ivory waxy lilac indurated, salt pepper dyspigmentation,
face- limited mouth opening furrows
hand- edema,CREST Calcinosis Raynauds digital infarct; nailfold capillaries Sclerodactylyl(pray)

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7
Q

scleroderma systemic

A
  1. muscle motility: dysphagia, proximal myopathy,
  2. BP( renal crisis), ILD-dry cough, fine creps /pulmonary HT( exertional dysnpea, JVP)
  3. overlap
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8
Q

Overlap syndromes/MCTD- kusukawa criteria

A

(SLE, DMS) raynauds, finger/hand swelling/acral sclerosis+U1 RNP, antifibrillin, antiPMScl + 1 feature

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9
Q

morphea classX

A

Cx: plaque/guttate/generalised(>2 sites); bullous, linear, deep

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10
Q

post strep scleredema

A

(acute cervicofacial hardening ,mask facies, limited mouth opening/swallowing)

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11
Q

scleredema monoclonal gammopathy,

A

persisitent;

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12
Q

scleredema diabeticorum

A

peau d orange erythema induration, waxy distal extremities

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13
Q

papular mucinosis-stigmata , subtypes

A

clusters/linear/close spaced; flesh/waxy papules
discrete papular/acral,HIV; self healing, nodular,
+ sclerodermoid - scleromyxedema

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14
Q

GVHD ClassX

A

traditional : 100d post SCT

NIH- organ specific criteria, diagnostic skin *

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15
Q

aGVHD:

A

Fx: morbilliform, follicular, petechial, acral, (st 4)TEN like 4-6w post HSCT;
staging: BSA, GIT/liver, histo

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16
Q

cGVHD

A

Fx: 1)LP like: reticulate pink-violet scaly papules plaques/mucosal/nail(dorsal pterygium, anocyhia)

2) papulosquamous psoriasiform/KP follicular erythema/SCLE
3) poikiloderma 4) sclerodermoid (patchy, diffuse symmetric); EF- rippled irregular nodular , groove sign

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17
Q

LSA Fx+ cX?

A
  • Atrophic ivory white wrinkled , obliterated labia majora minora introitus/meatus, cannot retract foreskin, hemorrhage, erosive; lilac margin(Extragenital)
  • irreversible scarring/stenosis; malignancy(non healing ulcers fissures nodules)
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18
Q

nail LP

A

longtudinal ridging, depressions, pterygium subungual hyperkeratosis

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19
Q

Pancreatic panniculitis-

A

ulcerated oily discharging nodule; fever abdo pain

Schmids triad- subcut nodules, polyarthritis(lytic) eosinophilia

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20
Q

SPTL

A

recurrent subcutaneous lesions fever, night sweats, fatigue and weight loss,
Cx: Ulceration, haemophagocytic syndrome(pancytopenia, prolonged fever, aggressive)

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21
Q

PRP CLASSx

A

Subtypes: 1)classical adult, 50%, cephalocaudal, orange red islands sparing, follicular hyperkeratosis, remit 3 y 2)atypical,icthyosiform chronic 3) classic juvenile 10% , 1 y remission

4) 25% circumscribed uncertain prognosis,
5) atypical juvenile, follicular hyperkeratosis familial CARD14, 5%
6) HIV associated( +follicular occlusion triad, lichen spinulosus like,HAART, poor prognosis)

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22
Q

palmar pits:

A

palmar pits:circular few mm, perpendicular edges; Approach: 1-; 2- keratoses detachment

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23
Q

PPK

A

calloused palms,soles,fingertips, hypothenar,thenar eminences ;plantar fissuring, thickening weight bearing areas, palmar pits?

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24
Q

PC CLASSx

A

Focal +associated :Pachyonychia congenita type 1: Jadassohn–Lewandowsky type;2- Jackson–Lawler type; new:K6a/b/c/16/17

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25
EB pruriginosa
: prurigo like,violaceous linear scarring,trauma blistering, milia,nail dystrophy
26
hypertrophic LP
(symmetric dorsal hyperkeratotic SCC)
27
perforating GA generalised-
DM, flesh-red colored grouped, central crust/umbilicated
28
lichen macular amyloid MEN2a-
sipple ripples,AD RET thyroid medullary ca, phaechromo
29
anetoderma-
circumscribed areas 1-2cm:flaccid, depressions, wrinkling, sac-like protrusions, skin-white blue, buttonhole sign, chest/back/neck/upper extremities
30
Degos-
porcelain white atrophic papules+central necrosis, erythema/telangiectasia, thrombotic vasculopathy:
31
EED,
Extensors myeloma, IgA monoclonal gammopathy , MDS, HIV
32
cowden’s
10queing COWs in a PtEN MOOing(trichilemomma) on COBBLES(tone) on TOES(acral keratoses, pits) - hyperactive(thyroid), Breastfeeding(adenomas), diarrhea(GIpolyp) menstruating(endometrium)
33
TS
(AD, TSC1/2, Ashleaf, CALM,shagreen(CTN),angiofibromas/collagenomas-Fibroma-gingival/subungual , EPilepsy, Low Intelligence, Angiofibroma( renal angiomyolipoma, pulm lymphangioleiomatosis, cardiac rhabdomyoma)
34
Muirr torre syndrome
(AD MLH1/MSH2 DNA mismatch repair, MSI; colon/GU/lung.breast CA)
35
EGFR inhibitor( cetuximab, erlotinib, gefitinib)-
CLR/breast/pancreatic/nonsmall cell lung; papulopustular, tetracycline-responsive ,aw xerosis, brittle hair paroncyhia, PG
36
NLD
symmetric pretibial sclerodermatous plaque red-brown circumscribed elevated rim→yellow periphery central atrophy/telangiectasis hypoanesthesia, alopeciaSCC
37
cutaneous rosai dorfman -
a/w :10% skin fever, +-massive painless bilateral cervical lymphadenopathy extranodal-soft tissue, eye, salivary gland, URT, CNS, bone, immune dysfunction
38
xanthoma disseminatum-
mucocutaneous xanthoma, DI, flexural
39
EPS
(rosegray sinuous, neck face, flexural;elastic;penicillamine)
40
eosinophilic follicultis:
(follicular inflammatory infiltrate-eosinophils)
41
Ofuji
-recurrent crops papules plaques studded sterile follicular pustules, annular, central clearaing, 50% pruritus/peripheral eos;
42
2. HIV follicultis:
(pruritic, truncal, papular)/hematologic malignancy assoc
43
alopecia mucinosa
(children/young adult) alopecia, prominent follicular opening, idiopathic vs lymphoma associated(older, widespread)
44
men2B
-(blubbery lips+ thyroid medullary CA+phaechromocytoma)
45
Melkersson-Rosenthal syndrome
recurrent swelling lips, Facial palsy (muscle weakness)tongue fissuring
46
rhinoscleroma- INVX
``` Klebsiella rhinocslceromatosis(MacConkey agar) warthin starry(G-ve intra/extracellular rod) mikulicz cells, russel bodies, ```
47
rhinoscleroma-
clinical: catarrhal-atrophic(obstructive purulent rhinorrhea), granulomatous(bony destruction, LN),
48
IGD-
arthritis, annular plaque linear cord trunk axilla medial thigh, RA
49
PNGD-
umbilicated papule(extensor, symmetrical)RA SLE, Wegener, LCV, vasculitis, palisading granuloma
50
Follicular atrophoderma- KP atrophicans spectrum
follicular papules, pitted atrophic depressions, vermiculate reticulate pattern, scarring alopecia scalp eyebrow/lashses
51
trichodysplasia spinulosa
(spiny projection,polyoma virus, ,LM spicule test, immunosuppressant, antiviral)
52
1Atrophoderma vermiculatum
-cheeks, preauricular, upperlip aw : sporadic, AD, sporadic, rombo(milia, telangiectasia, BCC/TE; bazex, - multiple BCC, nicholas balus, tuzun(fissured tongue) CHH(blaschkoid follicular atrophoderma)
53
ulerythema ophryogenes-
lateral1/3 eyebrows(scarring alopecia), temples cheeks | aw: Noonan, Conelia de lange
54
.keratosis folliculitis spinulosa decalvans-
scarring alopecia -scalp eyebrow/lash+ PPK, photophobia
55
LPP graham little syndrome
–lichenoid follicular eruption, scalp scarring alopecia, non scarring alopecia axilla public hair ;
56
piezogenic pedal papules-
skin soft plantar medial soles wt bearing/disappear elevation, orthotics/Sx
57
EDV
-(TV like-pigment; verrucuous-hands feet face);
58
cutaneous plasmacytosis:
LN+HSM, FBC/ESR, total protein serum electrophoresis(hypergammaglobulinemia), urine-bence jones( MM), constitutional,
59
antimalarial pigmentation
blue black grey | pretibial face hard palate subungual
60
minocycline pigmentation
(scars, LL, fe granules/melanin);
61
erythema dyschromicum perstans:pruritc erythema
->grey macules,halo?symmetric, trunk, proximal extremities, melanophages+melanin incontinence; HIV/NH4No2)
62
LPP
:type ⅘ phototype, papules,ill-defined oval,confluent; slate-grey;diffuse, reticulate, blotchy; face neck sunexposed; basal vac+melanophages; henna/dye/fragrance)
63
SAPHO
-synovitis, acne conglobata, pustulosis, hyperostosis(thickening/growth), osteitis
64
Sneddon wilkinson’s
IgA paraproteinemia dapsoneR
65
IgA pemphigus-
(subcorneal pustulosis-Ig A ab against desmocollin,intraepidermal neutrophilic-sunflower configuration)-
66
SDRIFE
- 1)systemic drug exposure(hours-d),2)sharply-demarcated erythema buttocks/thighs 3) +1 flexure 4)symmetry 5)absent systemic,
67
TEC
painful acral/intertriginous erythema-dusky-petechiael/edema/bullae+ desquamative gingivitis/gut; 2-3w postchemoRx(cytarabine, doxorubicin, 5FU, doclitaxel, MTX)
68
neutrophilic eccrine hidradenitis ,
Periorbital- AML, perieccrine neutrophils
69
Reiter’s
circinate balanitis,keratoderma blenorrhagica/psoriasiform rash/nail, arthritis, uveitis/iritis+ urethritis);
70
angiokeratoma mibelli-
warty-distal limb, fordyce-scrotum/vulva blue red;
71
multiple : angiokeratoma corporis diffusum
-symmetrical bathing trunk | Fabry’s fucosidosis
72
Fabry’s -
XLR , a-galactosidase A deficiency,lysosomal storage, urine birrefringent maltese cross(lipid globules), +/-mucosa,hypohidrosis, HOCM, NL(pain+ paresthesia, cerebrovascular),corneal opacities prog CRF,
73
fucosidosis
sinopulmonary, organomegaly, dysostosis multiplex)
74
glomeruloid hemangioma/cherry angioma
Polyneiropathy, Organomegaly, Endocrinopathy M-protein, Skin changes-hyperpig/trichosis/hidrosis/scleroderma/clubbing+leukonychia aw myeloma, castleman, plasmacytoma)
75
Arsenic
-ROS, altered DNA methylation, bowens, NMSC,↑internal malignancy(lung bladder/prostate CA(smokers), GIT/liver)
76
Organ transplant NMSC Risk
(swedish 2013 ,accelerating x300(H/L) X200(renal) ; korea 2014 x26)
77
Gorlin:
1) >5/early-onset(2, odontogenic keratocyst, 2) dysmorphism(frontal bossing hypertelorism);falx lamellar calcification 3) 1deg relative macrocephaly/cleft lip palate/bifid vertebrae/polydactylyl medulloblastoma/meningioma/ocular, ovarian fibroma
78
Bazex:
XLD, follicular atrophoderma, milia, hypotrichosis/hidrosis
79
Annular elastotic giant cell granuloma-
central clearing centrifugal extension, elastolysis elastophagocytosis,
80
HIES
IgE>1000, cold abscess, purulent pneumonia, | Skeletal(coarse, broad nasal,bossing, osteopenia), lymphoma
81
WAS-
1. ↑IgE/IgA,↓IgM/IgG, recurrent pyogenic infx | 2. bleeding ↓plr, 3. persistent eczema
82
CGD-
abn NBT reduction, phagocyte failure+granuloma formation
83
buschke ollendorffsyndrome
connective tissues nevi
84
Goltz
( telangiectasia, vermiculate dermal atrophy)
85
anetoderma-Primary-
Jadassohn(preceding inflammatory)vs Shweninger(absent)/familial
86
anetoderma-Secondary
inflammatory dermatosis(site of previous)— 1) drug-penicillamine, 2) infection-VZV folliculitis,TB,syphilis/tumor-autoimmune(APS)/HIV; 3) DF, involuted hemangioma

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